Marfan & Related Disorders

Nervous System

The nervous system – the brain and spinal column – are surrounded by fluid contained in a membrane called the dura, which is primarily made up of connective tissue. The enlargement of this membrane (and sometimes the presence of cysts) is referred to as dural ectasia.

Dural ectasia is present in more than 60 percent of people who have Marfan syndrome. The presence of dural ectasia does not always cause problems, although in some people it causes back, abdominal, and leg pain and headaches.

Dural ectasia is best identified through MRI imaging, particularly of the lower spine with a person standing upright. A mylogram, CT scan, or plain spinal films might also reveal the presence of dural ectasia. Here are some frequently asked questions about dural ectasia.

Does dural ectasia occur only in the lower spine?

No, dural ectasia can also occur in the neck or in the upper torso, but this is very rare. In 99 percent of people with dural ectasia, it occurs in the lowest part of the spine because this is where the fluid pressure is greatest when standing.

Can trauma such as an automobile accident cause dural ectasia in people with Marfan syndrome?

Yes, dural ectasia can present itself following trauma, but it doesn’t commonly occur this way.

What are the symptoms of dural ectasia and how are they treated?

The symptoms of dural ectasia vary. They include aching in the very low back, almost in the tailbone, as well as abdominal pain, headaches, and leg pain. Pain and numbness in the perineum (the area between the legs in front of the anus) can also occur.

When should symptoms of dural ectasia be evaluated?

The need for an evaluation depends on the degree of symptoms and disability. If the symptoms are tolerated, there is no urgency to be evaluated.

Are there other spinal problems that are associated with dural ectasia?

Dural ectasia does thin the spinal vertebrae, which can have implications for a person having surgery on the spine for any reason.

Is it safe to have a spinal MRI if titanium rods are in place due to scoliosis?

A person can have an MRI with any kind of spinal rod. There may be a "halo" or invisible area on the MRI in the immediate area around the rod. Titanium creates the smallest halo.